• Tuberculosis and Respiratory Diseases
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• v.40 no.2
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• pp.197-202
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• 1993

Polymyositis (PM) is a inflammatory connective tissue disease involving predominantly skeletal muscles, characterized by symmetrical, proximal muscle weakness, inflammation, and frequently, degeneration. Interstitial lung disease in association with PM occurs in 5~10% of cases and carries an especially grave prognosis. Although the cause of lung involvement in PM is not known, the underlying pathologic process in the lung is an immune mediated inflammation of alveolar structures, alveolitis. It is of interest, therefore, that cyclophosphamide, an immune modulating agent, has been reported to be effective in the treatment of PM. We report a case of corticosteroid resistant PM associated with interstitial lung disease, successfully treated with cyclophosphamide. A 37-year-old female was presented with 8 months duration of cough, exertional dyspnea, and muscle weakness. She had typical symptoms, physical findings, and elevated muscle enzyme levels in serum with characteristic findings of muscle biopsy. She also had typical interstitial lung disease pattern on chest X-ray and high resolution CT with restrictive pattern on pulmonary function test. The findings of transbronchial lung biopsy was compatible with interstitial lung disease. She failed to respond to corticosteroid initially. Subsequently steroids and cyclophosphamide were given with excellent clinical improvement.

• Tuberculosis and Respiratory Diseases
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• v.76 no.2
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• pp.75-79
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• 2014

Trichloroethylene (TCE) is a toxic chemical commonly used as a degreasing agent, and it is usually found in a colorless or blue liquid form. TCE has a sweet, chloroform-like odor, and this volatile chlorinated organic chemical can cause toxic hepatitis, neurophysiological disorders, skin disorders, and hypersensitivity syndromes. However, the hypersensitivity pneumonitis (HP) attributed to TCE has rarely been reported. We hereby describe a case of HP associated with TCE in a 29-year-old man who was employed as a lead welder at a computer repair center. He was installing the capacitors on computer chip boards and had been wiped down with TCE. He was admitted to our hospital with complaints of dry coughs, night sweats, and weight losses for the past two months. HP due to TCE exposure was being suspected due to his occupational history, and the results of a video-associated thoracoscopic biopsy confirmed the suspicions. Symptoms have resolved after the steroid pulse therapy and his occupational change. TCE should be taken into consideration as a potential trigger of HP. Early recognition and avoidance of the TCE exposure in the future is important for the treatment of TCE induced HP.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.28-34
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• 1992

Background: Diffuse panbronchiolitis (DPB) is a chronic obstructive pulmonary disease distinguished from bronchial asthma, chronic bronchitis, pulmonary emphysema, bronchiectasis, or alveolitis. It is prevalent in Japan, but is known to be rare outside of Japan. Only a few cases in Chinese, Italian, Noirth American and Korean have been reported. During recent two years we have found 16 cases of DPB in Seoul National University Hospital and analyzed the clinical characteristics. Method: We reviewed the patients' age, sex, duration of illness, smoking history, occupational history and past medical history. And we analyzed patients' symptoms, physical signs, laboratory findings and responses to treatment. Results: 1) Male: female ratio was 2.2:1, the ages ranged from 27 to 72 years old, and the duration of disease varied from 1 to 20 years. 2) Most of the patients were being treated as bronchiectasis, miliary tuberculosis, chronic bronchitis or bronchial asthma before they were diagnosed as DPB. 3) Only one patient was a smoker, and 25% of patients had the occupational history of exposure to particles or gas. And all patients had paranasal sinusitis. 4) High resolution computed tomography (HRCT) was very useful in diagnosing the disease in most of the patients. 5) PFT showed obstructive and restrictive abnormalities, and blood gas revealed hypoxemia in most of the patients. 6) Pseudomonas aeruginosa was isolated in the sputum of four patients (25%). 7) The titers of cold hemaglutinin, RA factor and CRP were elevated in most of the patients. 8) Most of the patients improved after treatment with erythromycin. Conclusion: DPB might not be a rare disease in Korea. So DPB should be suspected in a patient who has chronic cough, sputum, dyspnea and diffuse fine nodular chest X-ray abnormality. HRCT and open lung biopsy should be considered in a patient suspected of DPB.

• Tuberculosis and Respiratory Diseases
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• v.41 no.2
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• pp.127-134
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• 1994

Background : Diffuse panbronchiolitis(DPB) is a chronic inflammatory lung disease of unknown etiology which has characteristic clinical, radiological and pathological features, and is distinguished from bronchial asthma, chronic bronchitis, pulmonary emphysema, bronchiectasis, or alveolitis. Clinically, patients with DPB have chronic cough, purulent sputum, exertional dyspnea, and finally respiratory failure. Until a few years ago, the prognosis of DPB had been thought to be very grave, because there had been no effective treatment for the disease. But recent1y, low-dose long-term erythromycin was found to be very effective on DPB. Even though DPB is prevalent in Japan, and is known to be rare outside of Japan, we have a1ready reported the clinical features of 16 DPB cases in Korea. We tried low-dose long-term erythromycin on DPB patients and analyzed the clinical effect of erythromycin. Methods : We analyzed the changes of subjective symptoms, physical signs, pulmonary function tests and chest X-rays on 14 DPB patients with more than 6 months erythromycin treatment during the period from September 1989 to August 1992 in Seoul National University Hospital. Results : 1) Subjective symptoms improved in all patients within 2-3 months, and 54.5% of the patients showed no symptom after one year of treatment. 2) Crackles and wheezing decreased in 92.9% of the patients after 3 months and completely disapppeared in 63.5% of the patients after one year of treatment. 3) FVC and FEV1 increased remarkably during the first 3 months, and slowly increased thereafter, reaching normal level after one year of treatment. 4) Small nodular lesions on chest X-ray decreased in all patients, and chest PA was normal in 36.4% of the patients after one year of treatment. 5) There was side effect in one patient, stopping medication because of dyspepsia. One patient stopped medication because of no symptom after 16 months of treatment, but her symptom recurring after one month, improving again after retreatment. Conclusion: Low-dose long-term erythromycin showed ramarkable effectiveness on DPB. Further studies are needed on the mechanism of the drug and the duration of the treatment.

• Tuberculosis and Respiratory Diseases
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• v.44 no.4
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• pp.871-888
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• 1997

Background : It is now thought that the earliest manifestation of idiopathic pulmonary fibrosis is alveolitis, that is, an accumulation of inflammatory and immune effector cells within alveolar walls and spaces. Inflammatory cells including alveolar macrophages and resident normal pulmonary tissue cells participate through the release of many variable mediators such as inflammatory growth factors and cytokines, which contribute to tissue damage and finally cause chronic pulmonary inflammation and fibrosis. This study was performed to investigate the source and distribution pattern of transforming growth factor-${\beta}_1$(TGF-${\beta}_1$), platelet derived growth factor(PDGF), basic fibroblast growth factor(bFGF), interleukin 1(IL-1), interleukin 6(IL-6), tumor necrosis factor-$\alpha$ (TNF-$\alpha$) and the role of these mediators on bleomycin(BLM)-induced pulmonary injury and fibrosis in rats. Method : Wistar rats were divided into three groups(control group, BLM treated group, BLM and vitamine E treated group). Animals were sacrificed periodically at 1, 2, 3, 4, 5, 7, 14, 21, 28 days after saline or BLM administration. The effects were compared to the results of bronchoalveolar lavage fluid analysis, light microscopic findings, immunohistochemical stains for six different mediators(TGF-${\beta}_1$, PDGF, bFGF, IL-1, IL-6 and TNF-$\alpha$) and mRNA in situ hybridization for TGF-${\beta}_1$. Results : IL-1 and IL-6 are maximally expressed at postbleomycin 1~7th day which are mainly produced by neutrophils and bronchiolar epithelium. It is thought that they induce recruitment of inflammatory cells at the injury site. The expression of IL-1 and IL-6 at the bronchiolar epithelium within 7th day is an indirect evidence of contribution of bronchiolar epithelial cells to promote and maintain the inflammatory and immune responses adjacent to the airways. TNF-$\alpha$ is mainly produced by neutrophils and bronchiolar epithelial cells during 1~5th day, alveolar macrophages during 7~28th day. At the earlier period, TNF-$\alpha$ causes recruitment of inflammatory cells at the injury site and later stimulates pulmonary fibrosis. The main secreting cells of TGF-${\beta}_1$ are alveolar macrophages and bronchiolar epithelium and the target is pulmonary fibroblasts and extracellular matrix. TGF-${\beta}_1$ and PDGF stimulate proliferation of pulmonary fibroblasts and TGF-${\beta}_1$ and bFGF incite the fibroblasts to produce extracellular matrix. The vitamine E and BLM treated group shows few positive cells(p<0.05). Conclusion : After endothelial and epithelial injury, the neutrophils and bronchiolar epithelium secrete IL-1, IL-6, TNF-$\alpha$ which induce infiltration of many neutrophils. It is thought that variable enzymes and $O_2$ radicals released by these neutrophils cause destruction of normal lung architecture and progression of pulmonary fibrosis. At the 7~28th day, TGF-${\beta}_1$, PDGF, bFGF, TNF-$\alpha$ secreted by alveolar macrophages sting pulmonary fibroblasts into proliferating with increased production of extracellular matrix and finally, they make progression of pulmonary fibrosis. TNF-$\alpha$ compares quite important with TGF-${\beta}_1$ to cause pulmonary fibrosis. Vitamine E seems to decrease the extent of BLM induced pulmonary fibrosis.

• Tuberculosis and Respiratory Diseases
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• v.43 no.1
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• pp.75-87
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• 1996

Background: Lung cancer is the second most frequent malignancy in man in Korea. Surgery is the best treatment modality for non-small cell lung cancer, but most patients were presented in far advanced stage. So radiation therapy(RT) with or without chemotherapy is the next choice and radiation-induced pneumonitis and pulmonary fibrosis is the major limiting factor for the curative RT. Radiation pneumonitis is manifested with fever, cough and dyspnea, 2~3 months after the termination of radiotherpy. Chest X ray shows infiltration, typically limited to the radiation field, but occasionally bilateral infiltration was reported. Also Gibson et al reported that BAL lymphocytosis was found in both lungs, even though the radiation was confined to one lung. The aim of this study is to investigate the change of adhesion molecules expression on BAL cells and serum soluble ICAM-1(sICAM-1) level after the RT and its relationship to the development of radiation pneumonitis. The second aim is to confirm the bilaterality of change of BAL cell pattern and adhesion molecule expression. Subjects: BAL and the measurement of sICAM level in serum and BALF were done on 29 patients with lung cancer who received RT with curative intention. The BAL was done before the RT in 16 patients and 1~2 month after RT in 18 patients. 5 patients performed BAL before and after RT. Result: Clinically significant radiation pneumonitis developed in 7 patients. After RT, total cell count in BAL was significantly increased from $(20.2{\pm}10.2){\times}10^6\;cells/ml$ to $(35.3{\pm}21.6){\times}10^6\;cells/ml$ (p=0.0344) and %lymphocyte was also increased from $5.3{\pm}4.2%$ to $39.6{\pm}23.4%$ (p=0.0001) in all patient group. There was no difference between ipsilateral and contraleteral side to RT, and between the patients with and without radiation-pneumonitis. In whole patient group, the level of sICAM-1 showed no significant change after RT(in serum: $378{\pm}148$, $411{\pm}150\;ng/ml$, BALF: $20.2{\pm}12.2$, $45.1{\pm}34.8\;ng/ml$, respectively), but there was a significant difference between the patients with pneumonitis and without pneumonitis (serum: $505{\pm}164$ vs $345{\pm}102\;ng/ml$, p=0.0253, BALF: $67.9{\pm}36.3$ vs $25.2{\pm}17.9\;ng/ml$, p=0.0112). The expression of ICAM-1 on alveolar macrophages (AM) tends to increase after RT (RMFI: from $1.28{\pm}0.479$ to $1.63{\pm}0.539$, p=0.0605), but it was significantly high in patients with pneumonitis ($2.10{\pm}0.390$) compared to the patients without pneumonitis ($1.28{\pm}0.31$, p=0.0002). ICAM-1 expression on lymphocytes and CD 18 (${\beta}2$-integrin) expression tended to be high in the patients with pneumonitis but the difference was statiastically not significant. Conclusion: Subclinical alveolitis on the basis of BAL finding developed bilaterally in all patients after RT. But clinically significant pneumonitis occurred in much smaller fraction and the ICAM-1 expression on AM and the sICAM-1 level in serum were good indicator of it.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.569-583
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• 1995

Background: The expression of the adhesion molecules on the cell surface is important in the movement of cells and the modulation of immune response. DILD starts as an alveolitis and progresses to pulmonary fibrosis. So adhesion molecules in these patients is expected to be increased. There are several reports about adhesion molecules in DILD in terms of the percentage of positive cells in immuno-stain, in which the interpretation is subjective and the data were variable. Methods: So we measured the relative median fluorescence intensity(RMFI) which is the ratio of the FI emitted by bound primary monoclonal antibody to FI emitted by isotypic control antibody of the cells in BALF of 28 patients with DILD(IPF:10, collagen disease:7, sarcoidosis:9, hypersensitivity pneumonitis:2) and 9 healthy control. Results: RMFI of the ICAM-1 on AM($3.30{\pm}1.16$) and lymphocyte($5.39{\pm}.70$) of DILD were increased significantly than normal control($0.93{\pm}0.18$, $1.06{\pm}0.21$, respectively, p=0.001, P=0.003). RMFI of the CD18 on lymphocyte was also higher($24.9{\pm}14.9$) than normal($4.59{\pm}3.77$, p=0.0023). And there was a correlation between RMFI of ICAM on AM and the % of AM(r=-0.66, p=0.0001) and lymphocyte(r=0.447, p=0.0116) in BALF. Also RMFI of ICAM on lymphocyte had a significant (r=0.593, p=0.075) correlation with the % of IL-2R(+) lymphocyte in BALF. The soluble ICAM(sICAM) in serum was also significantly elevated in DILD($499.7{\pm}222.2\;ng/ml$) compred to normal($199.0{\pm}38.9$) (p=0.00097) and sICAM in BAL fluid was also significantly higher than normal control group($41.8{\pm}23.0\;ng/ml$ vs $20.1{\pm}13.6\;ng/ml$). There was a Significant correlation between sICAM level in serum and the expression of ICAM-l on AM(r=0.554, p=0.0259).Conclusion: These data suggest that in DILD the expression of adhesion molecules is increased in the AM and BAL lymphocytes with elevated serum sICAM, and these parameter may be useful in determining disease activity.